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Single System Ectopic Ureteroceles with Anomalies of the Heart, Testis and Vas Deferens
0022-10347 /80/1231-0{)81$02 00/0 Vol. 12:J,
T1--1E ,}CUHNAL. OF UROLOGY
CopyTigbt © 1980 by
Printed in
WiUiams & Wilkins
DAVID K. ;roHNSON
AND
ALAN n PERLMUTTER
Children's n,,sz,m:u of }Michigan and Depariment of Urology, Wayne State University School Detroit, lvlichigan
ABSTRACT
included 6
2 vvith bifateral non-union of of hBaternJ_ atresia the vas defe.rens with abdominal testes.
defined as ureteroceles have
the lite:ratUYe we urete:roceles systen1 (tab!e cases series of ureteroceles or FINDINGS
TABLE 1
Sex (No.l""
References
Rott' BostroerE, E: Frnib, i. B. 1md Tiibing., ,!CB Mohr, 1884 Barth, W.: Cited in Ericsson 1 Mogg, R. A.: J. Urol., !/7; 1003, 1967 ,Johnston, J. H. and Johnson, L. M.: B:cit. J. Urol., 41: 61, 1969 Williams and Malek and as:;ue11aues Malek and Utz" Schulman'1 Ftu·kas and associates7 vV.: J. UmL, Brock, W. A. and Kaplan, 800, 1978 Prewitt and Lebovvjtz8 Present series
MATERIALS AND METHODS
t Malek, R. D
12 1 day
M (3) M (1), F
Ft
17
F
M ],/[ (4)
5 ?
F
2 11103,
M (S), F (1)
. Petsonal communication. TABLE
JJIAGNOEHS AND OPERATIVE TREATMENT
st1,1dies iricluded an excretory The most common
excimon
F M i\11
Age
Adult
* Male to female ratio 19:6.
found at tern;.ediate bet-vveen an
v.,.u,,wJum,
M M
Signs and Symptorns
No.
the ureterocele was
l-M
l day
Abdominal mass
2-M
3 mos.
l\lIJ.cmne111s. non-paltestes
3--M 4.-Tvl
3 yrs.
5-M 6--M
American .h,cademy
7--F 81
2 Associated Anomalies Atrial septa! defoct and ductus arte:riosus Ventricular sept.al defect, atresia of vas deferens, bHat. abdori1inal testes
2 )'TS.
4 yrs. 8 yrs. 10 yrs.
Non-union of bilat. abdominal testes of Fallot ab,cto1mJJ1al testis Ventricular septa] defect
82
JOHNSON AND PERLMUTTER
day-old newborn with an abdominal mass to a 10-year-old girl with persistent pyuria. Presenting symptoms or signs were related to obstruction or infection in only 2 children (table 2). Of the remaining 5 cases 3 were diagnosed during evaluation of non-palpable testes, 1 had abdominal pain and 1 had asymptomatic hydronephrosis that was discovered at cardiac catheterization for tetralogy of Fallot. Of the 2 patients with symptoms or signs referable to the urinary tract 1 had an abdominal mass (fig. 2, A) and 1 had
Fm. 3. Multiple calculi in ectopic ureterocele in case 7
persistent pyuria from multiple calculi in an ectopic ureterocele (fig. 3). Four patients had associated cardiac anomalies, 1 had recurrent intestinal intussusception and 3 had abdominal testes, 2 of which were bilateral. One boy (case 4) with a lesion having the features of an ectopic ureterocele and ectopic ureter also had unusual testicular and vas deferens anomalies similar to those in case 2. The ectopic ureter, serving as a multicystic dysplastic kidney, ended in a fusiform swelling beneath the trigonal mucosa before entering the seminal vesicle. The 2 boys with bilateral abdominal testes had bilateral nonunion of the vas deferens and testis, including 1 with bilateral atresia of the vas deferens. Figure 4 shows the left abdominal testis and abnormal gubernaculum without any epididymis in case 4. The vas deferens was found separately near the internal inguinal ring. Fm. 1. Radiologic findings of single system ectopic ureterocele include non-visualized right kidney and ipsilateral filling defect in bladder extending to bladder neck.
DISCUSSION
The embryology of a ureterocele and ureteral ectopia has
Fm. 2. Case 1. A, percutaneous pyelogram when patient was 2 days old. B, antegrade pyelogram when he was 1 year old. C, IVP 6 months after total reconstruction. Right kidney, although small, is well decompressed and left kidney shows residual pyelectasis.
83
SINGLE SYSTEM ECTOPIC URETEROCELES
had abdominal testes and testicular ductal anorn""'"'-"-'"'" 2 with non-union between the vasa and testes.
had bilateral
cases of deferens. i:i Male atresia of the vas deferens but the testes are scended. 14 ' 15 Other cases of bilateral atresia of the vas deforens have been discovered also had descended testes. IG, Case 5, u,,oc.;uv.:,c:u documents the value of a system. system ureters and '""''rP1"r><'»IPQ and the rarer system ureter without ureterocele' 8 · 19 are more common m REFERENCES l,
2.
epididymis in
a
ureteral bud
,.u,ccm,rnyv''-"'" duct results in a
1ninal uTeter and ureterocele
with mentioned that an terminate in the vas deferens or ,,,,,,,,""'-' 4).9
In 5 cases a
U'c'H'kUCHL,CHYH
systems, found 75 per cent of renal segments to be abnormal when associated with a similar to
may lead to abnormal ct1tte,rentia1t10,n The obstruction in an to stenosis the ureteral orifice or orifice in the urethxa vvhen the bladder neck is closed. An insult normal ""''"""'w, from 5 to 9 weeks may the caxdiac and anomalies associated with a ureterocele. The 7 weeks the intrnven, becomes formed. The association of duct with the rnte testis occurs at 9 weeks of
ureterocele in infants and child.ren; clinicaI 197: 1, 1954. der rechten Niere nebst einer Samenleiters der gleichen GeseUsch. in ')Vurzb. n. F., 125)
3. Williams, D. I, and Royle, M.: Ectopic ureter in the male child. Brit. J. UroL, 41: 421, 1969. 4. Malek, R. S., Kelalis, P, P., Burke, E, C, and Stickler, G. B.. Sirnple and ectopic ureternce!e in infancy and childhood. Surg., Gynec, & Obst., 134: 611, 1972. 5. Malek, R S. and Utz, D. C.: Crossed, fused, renal ectopia an ureterncele. J. Urol., Hl4: 665, 1970. 6. "'~"u,uuau, C. C.: Les implantations ectopiques de l'uretere, Acta. 40: 201, 1972. 7, ,J, and Firstater, M.: Crossed renal ectopia with ectopic ureterocele, J. UroL, 119: 836, 1978. 8, Jr. and Lebowitz, R L: The single ectopic ureter. 127: 941, 1976. 9. rp1·proc,,P1 and urethral ectopic ureters. In: ConMalformations of Anus and Edited by R Webster. 1963, a correlation of 10. J. UroL, l.14: 274, 1975, H, and Stephens, F. D.: The ureteric orifice: lL Mackie, G. G., the """" vcnu~,,.,; to radiologic status of duplex kidneys. Ped. Surg., 473, 1975. nn,og,mesrs of renal 12. Bernstein, J.: The (renal Ped. Clin. N. 13. J,V11c11,ei:su11, J.: of the ductus deferens and J. 14. A. D., Rule, A., Khaw, failure in males with fibrosis. New EngL J. 279: 65, 1968. 15. uvmucom D. S., Perlmutter, A, D., Jockin, H, and Shwachman, IL Genital abnormalities in male patients with cystic fibrosis, J. Urol., 106: 568, 1971. 16, Schmidt, S. S,: Co1ngenital absence of the ductus deferens. Lettel' to the Editor. 213: 2080, 1970. Amelas, H. D. and Dubin, L.: ~vu,,,"mou, absence of the ductus deferens. Letter to the Editor. 213: 2080, 1970. 18. R. A .. The single ectopic ureter. Brit. J, UrnL, 3, 1974. 19, ,Jcuuuuau, C. C,: Tbe single ectopic ureter, Eur. UroL, 2: 64, 1976.
T1--1E ,}CUHNAL. OF UROLOGY
CopyTigbt © 1980 by
Printed in
WiUiams & Wilkins
DAVID K. ;roHNSON
AND
ALAN n PERLMUTTER
Children's n,,sz,m:u of }Michigan and Depariment of Urology, Wayne State University School Detroit, lvlichigan
ABSTRACT
included 6
2 vvith bifateral non-union of of hBaternJ_ atresia the vas defe.rens with abdominal testes.
defined as ureteroceles have
the lite:ratUYe we urete:roceles systen1 (tab!e cases series of ureteroceles or FINDINGS
TABLE 1
Sex (No.l""
References
Rott' BostroerE, E: Frnib, i. B. 1md Tiibing., ,!CB Mohr, 1884 Barth, W.: Cited in Ericsson 1 Mogg, R. A.: J. Urol., !/7; 1003, 1967 ,Johnston, J. H. and Johnson, L. M.: B:cit. J. Urol., 41: 61, 1969 Williams and Malek and as:;ue11aues Malek and Utz" Schulman'1 Ftu·kas and associates7 vV.: J. UmL, Brock, W. A. and Kaplan, 800, 1978 Prewitt and Lebovvjtz8 Present series
MATERIALS AND METHODS
t Malek, R. D
12 1 day
M (3) M (1), F
Ft
17
F
M ],/[ (4)
5 ?
F
2 11103,
M (S), F (1)
. Petsonal communication. TABLE
JJIAGNOEHS AND OPERATIVE TREATMENT
st1,1dies iricluded an excretory The most common
excimon
F M i\11
Age
Adult
* Male to female ratio 19:6.
found at tern;.ediate bet-vveen an
v.,.u,,wJum,
M M
Signs and Symptorns
No.
the ureterocele was
l-M
l day
Abdominal mass
2-M
3 mos.
l\lIJ.cmne111s. non-paltestes
3--M 4.-Tvl
3 yrs.
5-M 6--M
American .h,cademy
7--F 81
2 Associated Anomalies Atrial septa! defoct and ductus arte:riosus Ventricular sept.al defect, atresia of vas deferens, bHat. abdori1inal testes
2 )'TS.
4 yrs. 8 yrs. 10 yrs.
Non-union of bilat. abdominal testes of Fallot ab,cto1mJJ1al testis Ventricular septa] defect
82
JOHNSON AND PERLMUTTER
day-old newborn with an abdominal mass to a 10-year-old girl with persistent pyuria. Presenting symptoms or signs were related to obstruction or infection in only 2 children (table 2). Of the remaining 5 cases 3 were diagnosed during evaluation of non-palpable testes, 1 had abdominal pain and 1 had asymptomatic hydronephrosis that was discovered at cardiac catheterization for tetralogy of Fallot. Of the 2 patients with symptoms or signs referable to the urinary tract 1 had an abdominal mass (fig. 2, A) and 1 had
Fm. 3. Multiple calculi in ectopic ureterocele in case 7
persistent pyuria from multiple calculi in an ectopic ureterocele (fig. 3). Four patients had associated cardiac anomalies, 1 had recurrent intestinal intussusception and 3 had abdominal testes, 2 of which were bilateral. One boy (case 4) with a lesion having the features of an ectopic ureterocele and ectopic ureter also had unusual testicular and vas deferens anomalies similar to those in case 2. The ectopic ureter, serving as a multicystic dysplastic kidney, ended in a fusiform swelling beneath the trigonal mucosa before entering the seminal vesicle. The 2 boys with bilateral abdominal testes had bilateral nonunion of the vas deferens and testis, including 1 with bilateral atresia of the vas deferens. Figure 4 shows the left abdominal testis and abnormal gubernaculum without any epididymis in case 4. The vas deferens was found separately near the internal inguinal ring. Fm. 1. Radiologic findings of single system ectopic ureterocele include non-visualized right kidney and ipsilateral filling defect in bladder extending to bladder neck.
DISCUSSION
The embryology of a ureterocele and ureteral ectopia has
Fm. 2. Case 1. A, percutaneous pyelogram when patient was 2 days old. B, antegrade pyelogram when he was 1 year old. C, IVP 6 months after total reconstruction. Right kidney, although small, is well decompressed and left kidney shows residual pyelectasis.
83
SINGLE SYSTEM ECTOPIC URETEROCELES
had abdominal testes and testicular ductal anorn""'"'-"-'"'" 2 with non-union between the vasa and testes.
had bilateral
cases of deferens. i:i Male atresia of the vas deferens but the testes are scended. 14 ' 15 Other cases of bilateral atresia of the vas deforens have been discovered also had descended testes. IG, Case 5, u,,oc.;uv.:,c:u documents the value of a system. system ureters and '""''rP1"r><'»IPQ and the rarer system ureter without ureterocele' 8 · 19 are more common m REFERENCES l,
2.
epididymis in
a
ureteral bud
,.u,ccm,rnyv''-"'" duct results in a
1ninal uTeter and ureterocele
with mentioned that an terminate in the vas deferens or ,,,,,,,,""'-' 4).9
In 5 cases a
U'c'H'kUCHL,CHYH
systems, found 75 per cent of renal segments to be abnormal when associated with a similar to
may lead to abnormal ct1tte,rentia1t10,n The obstruction in an to stenosis the ureteral orifice or orifice in the urethxa vvhen the bladder neck is closed. An insult normal ""''"""'w, from 5 to 9 weeks may the caxdiac and anomalies associated with a ureterocele. The 7 weeks the intrnven, becomes formed. The association of duct with the rnte testis occurs at 9 weeks of
ureterocele in infants and child.ren; clinicaI 197: 1, 1954. der rechten Niere nebst einer Samenleiters der gleichen GeseUsch. in ')Vurzb. n. F., 125)
3. Williams, D. I, and Royle, M.: Ectopic ureter in the male child. Brit. J. UroL, 41: 421, 1969. 4. Malek, R. S., Kelalis, P, P., Burke, E, C, and Stickler, G. B.. Sirnple and ectopic ureternce!e in infancy and childhood. Surg., Gynec, & Obst., 134: 611, 1972. 5. Malek, R S. and Utz, D. C.: Crossed, fused, renal ectopia an ureterncele. J. Urol., Hl4: 665, 1970. 6. "'~"u,uuau, C. C.: Les implantations ectopiques de l'uretere, Acta. 40: 201, 1972. 7, ,J, and Firstater, M.: Crossed renal ectopia with ectopic ureterocele, J. UroL, 119: 836, 1978. 8, Jr. and Lebowitz, R L: The single ectopic ureter. 127: 941, 1976. 9. rp1·proc,,P1 and urethral ectopic ureters. In: ConMalformations of Anus and Edited by R Webster. 1963, a correlation of 10. J. UroL, l.14: 274, 1975, H, and Stephens, F. D.: The ureteric orifice: lL Mackie, G. G., the """" vcnu~,,.,; to radiologic status of duplex kidneys. Ped. Surg., 473, 1975. nn,og,mesrs of renal 12. Bernstein, J.: The (renal Ped. Clin. N. 13. J,V11c11,ei:su11, J.: of the ductus deferens and J. 14. A. D., Rule, A., Khaw, failure in males with fibrosis. New EngL J. 279: 65, 1968. 15. uvmucom D. S., Perlmutter, A, D., Jockin, H, and Shwachman, IL Genital abnormalities in male patients with cystic fibrosis, J. Urol., 106: 568, 1971. 16, Schmidt, S. S,: Co1ngenital absence of the ductus deferens. Lettel' to the Editor. 213: 2080, 1970. Amelas, H. D. and Dubin, L.: ~vu,,,"mou, absence of the ductus deferens. Letter to the Editor. 213: 2080, 1970. 18. R. A .. The single ectopic ureter. Brit. J, UrnL, 3, 1974. 19, ,Jcuuuuau, C. C,: Tbe single ectopic ureter, Eur. UroL, 2: 64, 1976.